What is peripartum cardiomyopathy?

Peripartum cardiomyopathy is a form of dilated cardiomyopathy that occurs during the last 3 months of pregnancy and up to 6 months postpartum, in the absence of a previous history of myocardial disorder. Endomyocardial biopsy reveals myocarditis in 30-50% of these cases.

The reported incidence of peripartum cardiomyopathy ranges from 1/3,000 to 1/15,000. The incidence is increased in women who are 30 years of age, hypertensive, toxemic, multiparous, and with twin pregnancies.

1A 50-year-old woman presents with worsening exertional dyspnea and lower-extremity edema. The physical examination demonstrates elevated jugular venous pressure, bilateral rales, an S3 gallop, and pitting edema of the lower extremities. She is obviously intoxicated. The chest x-ray shows an enlarged cardiac silhouette and a prominent interstitial pattern. What diagnostic possibilities should be considered?

The patient has congestive heart failure. Given that she is acutely intoxicated, further history should be obtained to assess for chronic alcoholism. If the patient is indeed alcoholic, the heart failure may be the result of alcoholic cardiomyopathy. Alcoholic cardiomyopathy is one of the most common secondary causes of dilated cardiomyopathy in the United States. Many patients improve markedly if they stop drinking.

Diagnostic thought should not end there though. Many chronic alcoholics eat poorly and develop multiple nutritional deficiencies. One of these, thiamine deficiency, can cause heart failure, so-called wet beriberi.‚ Most alcoholic patients with cardiomyopathy should be given a therapeutic trial of thiamine. In addition, ethanol does not provide protection against other diseases that cause cardiomyopathy, such as coronary artery disease.

1A 47-year-old man, who is tanned and appears healthy, presents with symptoms and signs of heart failure. On questioning, he reveals that he was diagnosed as borderline diabetic‚ at a screening examination several years ago, and that his father died suddenly in his late 40s. If you could order only one blood test for diagnostic purposes, what would it be?

Either a serum iron or ferritin, since it may give a clue to the presence of hemochromatosis. Hemochromatosis can cause heart failure, diabetes, and chronic liver disease. It is an important diagnosis to consider in patients with heart failure, because it is one of the few cardiomyopathies that may resolve with treatment. It is more common in men, is frequently familial, and typically presents in the fifth or sixth decade of life. A more aggressive form of the disease, primarily limited to the heart, may present at younger ages.

Describe the pathogenesis of restrictive cardiomyopathy.

Restrictive cardiomyopathy is the least common of the cardiomyopathies and is characterized by abnormal diastolic function in the presence of normal ventricular systolic function.

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